Table 1.
Summary of the cardiovascular roles, proteolytic functions and domain organization of the ADAMTS family. For the cardiovascular roles and diseases, the numbers in brackets indicate specific ADAMTS family members. CAD, coronary artery disease; PAH, pulmonary arterial hypertension; TAAD, thoracic aortic aneurysms and dissections; TTP, thrombotic thrombocytopenic purpura; VSMC, vascular smooth muscle cells; VWF, von Willebrand factor; WMS, Weill-Marchesani syndrome. The ADAMTS protein domains are abbreviated as follows: S, signal peptide; Pro, prodomain; Mp, metalloproteinase domain; Dis, disintegrin-like domain; CR, cysteine-rich domain; Sp, spacer domain; Muc, mucin-like domain; CUB, complement subcomponent C1r/C1s/embryonic sea urchin protein Uegf (urchin epidermal growth factor)/bone morphogenic protein 1; PL, protease and lacunin domain; GON, gon-1 like domain.
| proteolytic function | family members | domain organisation | cardiovascular role | disease |
|---|---|---|---|---|
| proteoglycanases | ADAMTS-1/15 |  |
regulation of PG turnover, LDL internalisation (5), development of cardiovascular system (1, 5, 9) | atherosclerosis (4) TAAD (1, 4, 5) PAH (8) |
| ADAMTS-4 |  |
|||
| ADAMTS-5/8 |  |
|||
| ADAMTS-9/20 |  |
|||
| procollagen N-propeptidases | ADAMTS-2/3/14 |  |
assembly of collagen fibrils myocardial repair (2) |
|
| unknown | ADAMTS-6/10 |  |
assembly of fibrillin microfibrils (10) | WMS (10) prolonged QRS (6) |
| unknown | ADAMTS-7/12 |  |
VSMC migration and re-endothelialisation (7) | CAD (7) |
| VWF-cleaving protease | ADAMTS-13 |  |
haemostasis | TTP, stroke |
| unknown | ADAMTS-16/18 |  |
regulation of blood pressure (16) | - |
| unknown | ADAMTS-17/19 |  |
assembly of fibrillin microfibrils (17) | WMS (17) heart valve disease (19) |